ABSTRACT
We present the case of a patient suffering from Neurofibromatosis type I (NF-1) with acute, very painful neuropathy of the right lower extremity. The preoperative electro neuro- physiological study showed an impaired function of the peroneal nerve. The MRI revealed an extended diffuse plexiform tumour of the sciatic nerve and at thigh level. Biopsies showed marked diffuse angiomatosis within the sciatic nerve.To our knowledge, this is the first description of an intraneural vascular malformation in NF-1.Treatment of such an entity is a challenge and must be individually defined.
Subject(s)
Angiomatosis/surgery , Neurofibromatosis 1/surgery , Sciatic Neuropathy/surgery , Angiomatosis/diagnosis , Angiomatosis/pathology , Diagnosis, Differential , Humans , Image Interpretation, Computer-Assisted , Magnetic Resonance Imaging , Male , Microsurgery/methods , Middle Aged , Neurofibromatosis 1/diagnosis , Neurofibromatosis 1/pathology , Sciatic Nerve/pathology , Sciatic Nerve/surgery , Sciatic Neuropathy/diagnosis , Sciatic Neuropathy/pathologyABSTRACT
PURPOSE: The etiology of Dupuytren disease is unclear. Pain is seldom described in the literature. Patients are more often disturbed by impaired extension of the fingers. We recently treated a series of patients who had had painful nodules for more than 1 year, and we therefore decided to investigate them for a possible anatomical correlate. METHODS: Biopsies were taken during surgery from patients with Dupuytren disease and stained to enable detection of neuronal tissue. RESULTS: We treated 17 fingers in 10 patients. Intraoperatively, 10 showed tiny nerve branches passing into or crossing the fibrous bands or nodules. Of 13 biopsies, 6 showed nerve fibers embedded in fibrous tissue, 3 showed perineural or intraneural fibrosis or both, and 3 showed true neuromas. Enlarged Pacinian corpuscles were isolated from 1 sample. All patients were pain free after surgery. CONCLUSIONS: Although Dupuytren disease is generally considered painless, we treated a series of early stage patients with painful disease. Intraoperative inspection and histological examination of tissue samples showed that nerve tissue was involved in all cases. The pain might have been due to local nerve compression by the fibromatosis or the Dupuytren disease itself. We, therefore, suggest that the indication for surgery in Dupuytren disease be extended to painful nodules for more than 1 year, even in the early stages of the disease in the absence of functional deficits, with assessment of tissue samples for histological changes in nerves.
